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Blood, 1 May 2008, Vol. 111, No. 9, pp. 4463-4470. Prepublished online as a Blood First Edition Paper on February 21, 2008; DOI 10.1182/blood-2007-08-105759.
CLINICAL TRIALS AND OBSERVATIONS Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials1 Inserm U728, Paris, France; Université Paris 7, U728, Paris, France; 2 Assistance Publique–Hôpitaux de Paris, Hôpital Saint-Louis, Service de Pathologie, Paris, France; 3 Service de Cancérologie, CHU de Nice l'Archet, Clinique Universitaire des Spécialités Médicales, Nice, France; 4 Service d'Hématologie adulte, Hôpital Saint Louis, Paris, France; 5 Service d'Hématologie, Hôpital Robert Debré, Reims cedex, France; 6 Institute for Pathology, University Clinic of Schleswig-Holstein (UK-SH), Lübeck, Germany; 7 Department of Pathology, Vrije Universiteit Medical Center (VUMC), Amsterdam, The Netherlands; 8 Service de Pathologie, Hôpital Ambroise Paré, Faculté de Médecine Boulogne cedex, France; 9 Service d'Hématologie, Institut Paoli-Calmettes, Marseille, France; 10 Service d'Hématologie, Université Catholique de Louvain, Mont Godinne, Yvoir, Belgium; 11 Service d'Anatomie Pathologique, Hôpital Hôtel Dieu, Paris, France; 12 Service d'Hématologie, Hôpital Henri Mondor, Créteil, France; 13 Service d'Hématologie, Pierre Bénite, France; 14 Inserm, U728, Paris, France; Université Paris 7, U728, Paris, France; AP-HP, Hôpital Saint-Louis, Service d'Hématologie, Paris, France; 15 Inserm U841, Créteil, France; Université Paris 12, Faculté de médecine, Créteil, France; AP-HP, Hôpital Henri Mondor, Département de Pathologie, Créteil, France To evaluate the prognostic significance of clinicobiologic and pathological features in angioimmunoblastic T-cell lymphoma (AITL), 157 AITL patients were retrieved from the GELA LNH87-LNH93 randomized clinical trials. One hundred forty-seven patients received a cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)–like regimen with intensified courses in half of them. Histologically, 41 cases were classified as "rich in large cells" and 116 as "classic" (including 19 rich in epithelioid cells, 14 rich in clear cells, and 4 with hyperplastic germinal centers). Sixty-two cases were scored for CD10 and CXCL13 expression according to the abundance of positive lymphoid cells. Median age was 62 years, with 81% advanced stage, 72% B symptoms, 65% anemia, 50% hypergammaglobulinemia, and 66% elevated LDH. Overall 7-year survival was 30%. In multivariate analysis, only male sex (P = .004), mediastinal lymphadenopathy (P = .041), and anemia (P = .042) adversely affected overall survival. Increase in large cells and high level of CD10 and CXCL13 did not affect survival. Intensive regimen did not improve survival. In conclusion, AITL is a morphologically heterogeneous T-cell lymphoma commonly expressing CXCL13 and CD10 and carrying few prognostic factors. It portends a poor prognosis even when treated intensively. However, AITL is not always lethal with 30% of patients alive at 7 years.
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